[CIS PIDD] [cis-pidd] Sweet syndrome associated with immune deficiency

[CIS-PIDD]

My suggestion is to evaluate as pyoderma  with his syndromic presentation of Autoinflammatory disease (neutrophilic infiltration and no response to T cell treatments) and try any anti cytokines (tnf, il6, 17 or il1). To the diagnsosis you can look for genes like pstpip1,  mefv (exons 2-4), lpin2 and cecr1.

Leo

Leonardo Oliveira Mendonça
Médico Especialista em Imunologia Clínica e Alergia, Doenças Autoimunes e Autoinflamatórias
Médico Especialista em Clínica Médica/Medicina Interna

Leonardo Oliveira Mendonça, MD
Specialist in Clinical Immunology and Allergy, Autoimmune and Autoinflammatory disorders
Consultant Specialist in Internal Medicine

E-mail: leonardo.oliveira.mendonca at gmail.com
Telefone/Phone Number: +55-11-3864-2246
Endereço/Adress: rua Heitor penteado, 477 - Sumarezinho - São Paulo - Brasil

> On Dec 19, 2016, at 6:50 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
> 
> … In these cases I would look for ADA2 activity (CECR1 mutations).
> … just a gut feeling, no evidence…
> Yours, Bodo
> 
> ****************************************
> Univ.-Prof. Dr. med. B. Grimbacher
>  
> Scientific-Director
> CCI-Center for Chronic Immunodeficiency
> UNIVERSITÄTSKLINIKUM FREIBURG
> Tel.: 0761 270-77731  Fax: -77744
> Breisacherstraße 115, 79106 Freiburg
> bodo.grimbacher at uniklinik-freiburg.de 
> www.uniklinik-freiburg.de/cci
> 
> 
> Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Datum: Monday 21 November 2016 14:56
> An: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Betreff: Re: [cis-pidd] Sweet syndrome associated with immune deficiency
> 
> I know a patient with CVID and Sweet Syndrome.
> 
> Yesim
> 
> 
> Yesim Yilmaz Demirdag, MD
> Columbia University Medical Center
> New York, NY
> (212) 305 2300
> 
> 
> 
>> On Sun, Nov 20, 2016 at 3:43 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
>> We have a 25 year old transgendered male with a history of severe Sweet’s syndrome that has flared multiple times despite immunosuppression including steroids, infliximab, cyclosporine, hyperbaric oxygen, dapsone and colchicine.  He requires frequent hospitalizations for flares and does respond to high dose IV steroids.  His Sweet’s syndrome has been attributed to his testosterone use as there is no other clear trigger (no malignancy, no IBD, no other drugs).  He has now been off testosterone for the past 3 months and continues to flare.  Given the severity of his Sweet’s syndrome, we were asked to evaluate whether there might be possible associated immune deficiency.  There are reports of Sweet’s syndrome associated with immune deficiency but there does not seem to be any clear association with any particular disorder but seems to be associated with chronic inflammation in the setting of immune deficiency.  He has been on significant immunosuppression so his testing is likely to be abnormal (we know he has hypogammaglobulinemia but in the setting of long term high dose steroids).  He has no history of infection prior to his diagnosis of Sweets about 15 months ago and no significant family history of infection, immune deficiency, or autoimmunity. 
>> 
>> Thanks,
>> 
>> Rebecca Saff
>> 
>>  
>> 
>> The information in this e-mail is intended only for the person to whom it is
>> addressed. If you believe this e-mail was sent to you in error and the e-mail
>> contains patient information, please contact the Partners Compliance HelpLine at
>> http://www.partners.org/complianceline . If the e-mail was sent to you in error
>> but does not contain patient information, please contact the sender and properly
>> dispose of the e-mail.
>> 
>> ---
>> 
>> You are currently subscribed to cis-pidd as: dryesimyilmaz at gmail.com.
>> 
>> To unsubscribe click here: http://cts.dundee.net/u?id=96396501.dcd0c9ab35d3ac44d4eef098bc4af6b8&n=T&l=cis-pidd&o=4006920
>> 
>> (It may be necessary to cut and paste the above URL if the line is broken)
>> 
>> or send a blank email to leave-4006920-96396501.dcd0c9ab35d3ac44d4eef098bc4af6b8 at lyris.dundee.net
>> 
> 
> ---
> 
> You are currently subscribed to cis-pidd as:  bodo.grimbacher at uniklinik-freiburg.de.
> 
> To unsubscribe click here: http://cts.dundee.net/u?id=96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a&n=T&l=cis-pidd&o=4008316
> 
> (It may be necessary to cut and paste the above URL if the line is broken)
> 
> or send a blank email to leave-4008316-96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a at lyris.dundee.net
> 
> ---
> 
> You are currently subscribed to cis-pidd as: leonardo.oliveira.mendonca at gmail.com.
> 
> To unsubscribe click here: http://cts.dundee.net/u?id=96396764.5d5926a8e01f475d7bf6717a52d027f0&n=T&l=cis-pidd&o=4077094
> 
> (It may be necessary to cut and paste the above URL if the line is broken)
> 
> or send a blank email to leave-4077094-96396764.5d5926a8e01f475d7bf6717a52d027f0 at lyris.dundee.net

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4077265
or send a blank email to leave-4077265-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20161219/1aa73ec8/attachment-0001.html>